Management of Adult Patients with Fontan Physiology

Abstract

The number of adult patients suffering late adverse outcomes after Fontan surgery, which is functional repair of congenital complex cyanotic heart disease with single ventricle physiology, has been steadily increasing. Fontan patients have diminished cardiac output and high central venous pressure, mainly due to absence of the pulmonary ventricle. Although adult patients are largely asymptomatic, their exercise capacity is reduced with excessive ventilation during exercise as in congestive heart failure. In addition, several late complications including arrhythmia, ventricular dysfunction, autonomic disturbance, liver fibrosis, and protein-losing enteropathy are known to occur in these patients. Therefore, there has been increased focus on health outcomes of adult patients late after Fontan surgery. Such patients need to be cared for using a multidisciplinary approach as in the case of patients with multi-organ disease, which comprises many interactions between the cardiovascular system and other organs.